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Kluver-Bucy syndrome

MedGen UID:
124361
Concept ID:
C0270707
Mental or Behavioral Dysfunction
Synonyms: Bilateral temporal lobe disorder; Memory loss, extreme sexual behavior, placidity, and visual distractibility; Post-encephalitic Kluver Bucy syndrome (type); Post-traumatic Kluver Bucy syndrome (type)
SNOMED CT: Klüver-Bucy syndrome (10651001)
 
Monarch Initiative: MONDO:0005817
Orphanet: ORPHA157823

Definition

A rare neurologic disease characterized by visual agnosia, hyperorality (strong tendency to examine objects orally), hypermetamorphosis (described as the irresistible impulse to notice and react to everything within sight), hypersexuality, changes in dietary habits and hyperphagia, placidity, and amnesia, due to bilateral lesions of the temporal lobe including the hippocampus and amygdala. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVKluver-Bucy syndrome
Follow this link to review classifications for Kluver-Bucy syndrome in Orphanet.

Professional guidelines

PubMed

Clay FJ, Kuriakose A, Lesche D, Hicks AJ, Zaman H, Azizi E, Ponsford JL, Jayaram M, Hopwood M
J Neuropsychiatry Clin Neurosci 2019 Winter;31(1):6-16. Epub 2018 Oct 31 doi: 10.1176/appi.neuropsych.18050112. PMID: 30376788
Mendez MF, Perryman KM
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Recent clinical studies

Etiology

Stewart E, Smith ML
Epilepsy Behav 2019 May;94:189-194. Epub 2019 Apr 7 doi: 10.1016/j.yebeh.2019.03.031. PMID: 30970297
Shapiro EG, Nestrasil I, Ahmed A, Wey A, Rudser KR, Delaney KA, Rumsey RK, Haslett PA, Whitley CB, Potegal M
Mol Genet Metab 2015 Apr;114(4):594-8. Epub 2015 Mar 5 doi: 10.1016/j.ymgme.2015.02.008. PMID: 25770355Free PMC Article
Kuang Y, Yang T, Gu J, Kong B, Cheng L
Br J Neurosurg 2014 Jun;28(3):374-7. Epub 2013 Oct 7 doi: 10.3109/02688697.2013.841854. PMID: 24099101
Potegal M, Yund B, Rudser K, Ahmed A, Delaney K, Nestrasil I, Whitley CB, Shapiro EG
J Clin Exp Neuropsychol 2013;35(6):608-16. Epub 2013 Jun 8 doi: 10.1080/13803395.2013.804035. PMID: 23745734Free PMC Article
Ozmen M, Erdogan A, Duvenci S, Ozyurt E, Ozkara C
Epilepsy Behav 2004 Feb;5(1):133-6. doi: 10.1016/j.yebeh.2003.10.009. PMID: 14751219

Diagnosis

Hernández-Martínez AE, Serrano-Juárez CA, Barrera-Medellín KG, Ramírez-Quiroga CI, Ramírez-Reyes AG, Casarrubias Islas R, Prieto-Corona B
J Neuropsychol 2024 Mar;18 Suppl 1:61-72. Epub 2023 May 4 doi: 10.1111/jnp.12320. PMID: 37139904
Hernandez-Vega M, Badial-Ochoa S, Rivas-Ruvalcaba FJ, Rodriguez-Leyva I
BMJ Case Rep 2022 Jul 11;15(7) doi: 10.1136/bcr-2022-250745. PMID: 35817482Free PMC Article
Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585
Khandelwal DC, Sethi PK, Anand I
J Assoc Physicians India 2005 Jan;53:23. PMID: 15857007
Clarke DJ, Brown NS
Br J Psychiatry 1990 Sep;157:439-41. doi: 10.1192/bjp.157.3.439. PMID: 2245280

Therapy

Jha KK, Singh SK, Kumar P, Arora CD
BMJ Case Rep 2016 Aug 16;2016 doi: 10.1136/bcr-2016-215926. PMID: 27530874Free PMC Article
Devinsky J, Sacks O, Devinsky O
Neurocase 2010 Apr;16(2):140-5. Epub 2009 Nov 18 doi: 10.1080/13554790903329182. PMID: 19927260
Daxboeck F
Curr Opin Neurol 2006 Aug;19(4):374-8. doi: 10.1097/01.wco.0000236617.04958.60. PMID: 16914976
Auvichayapat N, Auvichayapat P, Watanatorn J, Thamaroj J, Jitpimolmard S
Epilepsy Behav 2006 Feb;8(1):320-2. Epub 2005 Dec 13 doi: 10.1016/j.yebeh.2005.10.005. PMID: 16356778
Varga E, Haher EJ, Simpson GM
Biol Psychiatry 1975 Feb;10(1):65-8. PMID: 235332

Prognosis

Al-Attas AA, Aldayel AY, Aloufi TH, Biary N
J Med Case Rep 2021 Jul 15;15(1):346. doi: 10.1186/s13256-021-02932-0. PMID: 34261516Free PMC Article
Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585
Lauterbach EC
Psychiatr Clin North Am 2004 Dec;27(4):801-25. doi: 10.1016/j.psc.2004.07.001. PMID: 15550293
Ozmen M, Erdogan A, Duvenci S, Ozyurt E, Ozkara C
Epilepsy Behav 2004 Feb;5(1):133-6. doi: 10.1016/j.yebeh.2003.10.009. PMID: 14751219
Lanska DJ, Currier RD, Cohen M, Gambetti P, Smith EE, Bebin J, Jackson JF, Whitehouse PJ, Markesbery WR
Neurology 1994 Sep;44(9):1633-43. doi: 10.1212/wnl.44.9.1633. PMID: 7936288

Clinical prediction guides

Brigo F
Neurol Sci 2021 Sep;42(9):3939-3942. Epub 2021 Jan 26 doi: 10.1007/s10072-021-05070-z. PMID: 33495930
Caro MA, Jimenez XF
J Clin Psychiatry 2016 Aug;77(8):e982-8. doi: 10.4088/JCP.14r09497. PMID: 27380585
Lippe S, Gonin-Flambois C, Jambaqué I
Handb Clin Neurol 2013;112:1285-8. doi: 10.1016/B978-0-444-52910-7.00051-9. PMID: 23622339
Isaacson RL
Behav Cogn Neurosci Rev 2002 Jun;1(2):87-107. doi: 10.1177/1534582302001002001. PMID: 17715588
Gościński I, Kwiatkowski S, Polak J, Orłowiejska M
Acta Neurochir (Wien) 1997;139(4):303-6. doi: 10.1007/BF01808825. PMID: 9202769

Recent systematic reviews

Clay FJ, Kuriakose A, Lesche D, Hicks AJ, Zaman H, Azizi E, Ponsford JL, Jayaram M, Hopwood M
J Neuropsychiatry Clin Neurosci 2019 Winter;31(1):6-16. Epub 2018 Oct 31 doi: 10.1176/appi.neuropsych.18050112. PMID: 30376788

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